Segmental Darier’s disease: A case report
Segmental Darier’s disease is an uncommon subtype of Darier’s genodermatosis, resulting from a mutation in the ATPase type 2 during early embryogenesis. It typically presents as a persistent, pruritic papular eruption following the lines of Blaschko. Histopathology of Darier’s disease demonstrates a...
| Main Authors: | , |
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| Format: | Article |
| Language: | English |
| Published: |
SAGE Publishing
2023-04-01
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| Series: | SAGE Open Medical Case Reports |
| Online Access: | https://doi.org/10.1177/2050313X231160938 |
| _version_ | 1797852819139592192 |
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| author | Elena Pastukhova Lauren LaBerge |
| author_facet | Elena Pastukhova Lauren LaBerge |
| author_sort | Elena Pastukhova |
| collection | DOAJ |
| description | Segmental Darier’s disease is an uncommon subtype of Darier’s genodermatosis, resulting from a mutation in the ATPase type 2 during early embryogenesis. It typically presents as a persistent, pruritic papular eruption following the lines of Blaschko. Histopathology of Darier’s disease demonstrates acantholysis, dyskeratosis, and corps ronds. First-line treatment includes topical retinoids, calcineurin inhibitors, and synthetic vitamin D analogues. Severe disease may require systemic therapy with oral retinoids, immunomodulators, magnesium, and low-dose naltrexone. Segmental Darier’s disease is important to recognize both clinically and histologically as it may resemble other acantholytic Blaschkolinear dermatoses and should be considered in individuals presenting with a chronic localized papular eruption in a Blaschkoid distribution. Herein, we present a case of a 48-year-old male with segmental Darier’s disease who improved significantly following acitretin treatment. |
| first_indexed | 2024-04-09T19:39:20Z |
| format | Article |
| id | doaj.art-ede54431288f49b7a6f96694ff655b23 |
| institution | Directory Open Access Journal |
| issn | 2050-313X |
| language | English |
| last_indexed | 2024-04-09T19:39:20Z |
| publishDate | 2023-04-01 |
| publisher | SAGE Publishing |
| record_format | Article |
| series | SAGE Open Medical Case Reports |
| spelling | doaj.art-ede54431288f49b7a6f96694ff655b232023-04-04T09:04:21ZengSAGE PublishingSAGE Open Medical Case Reports2050-313X2023-04-011110.1177/2050313X231160938Segmental Darier’s disease: A case reportElena Pastukhova0Lauren LaBerge1Faculty of Medicine, University of Ottawa, Ottawa, ON, CanadaDivision of Dermatology, The Ottawa Hospital, University of Ottawa, Ottawa, ON, CanadaSegmental Darier’s disease is an uncommon subtype of Darier’s genodermatosis, resulting from a mutation in the ATPase type 2 during early embryogenesis. It typically presents as a persistent, pruritic papular eruption following the lines of Blaschko. Histopathology of Darier’s disease demonstrates acantholysis, dyskeratosis, and corps ronds. First-line treatment includes topical retinoids, calcineurin inhibitors, and synthetic vitamin D analogues. Severe disease may require systemic therapy with oral retinoids, immunomodulators, magnesium, and low-dose naltrexone. Segmental Darier’s disease is important to recognize both clinically and histologically as it may resemble other acantholytic Blaschkolinear dermatoses and should be considered in individuals presenting with a chronic localized papular eruption in a Blaschkoid distribution. Herein, we present a case of a 48-year-old male with segmental Darier’s disease who improved significantly following acitretin treatment.https://doi.org/10.1177/2050313X231160938 |
| spellingShingle | Elena Pastukhova Lauren LaBerge Segmental Darier’s disease: A case report SAGE Open Medical Case Reports |
| title | Segmental Darier’s disease: A case report |
| title_full | Segmental Darier’s disease: A case report |
| title_fullStr | Segmental Darier’s disease: A case report |
| title_full_unstemmed | Segmental Darier’s disease: A case report |
| title_short | Segmental Darier’s disease: A case report |
| title_sort | segmental darier s disease a case report |
| url | https://doi.org/10.1177/2050313X231160938 |
| work_keys_str_mv | AT elenapastukhova segmentaldariersdiseaseacasereport AT laurenlaberge segmentaldariersdiseaseacasereport |